Interstitial lung disease

Calendar Schedule

Book an Appointment

Diagnosis

Identifying and determining the cause of interstitial lung disease can be challenging. A large number of disorders fall into this broad category. In addition, the signs and symptoms of a wide range of medical conditions can mimic interstitial lung disease, and doctors must rule these out before making a definitive diagnosis.


Some of the following tests may be necessary.


Laboratory tests

Blood tests. Certain bloodwork can detect proteins, antibodies and other markers of autoimmune diseases or inflammatory responses to environmental exposures, such as those caused by molds or bird protein.

Imaging tests

Computerized tomography (CT) scan. This imaging test is key to, and sometimes the first step in, the diagnosis of interstitial lung disease. CT scanners use a computer to combine X-ray images taken from many different angles to produce cross-sectional images of internal structures. A high-resolution CT scan can be particularly helpful in determining the extent of lung damage caused by interstitial lung disease. It can show details of the fibrosis, which can be helpful in narrowing down the diagnosis and in guiding treatment decisions.

Echocardiogram. A sonogram for the heart, an echocardiogram uses sound waves to visualize the heart. It can produce still images of your heart's structures, as well as videos that show how your heart is functioning. This test can evaluate the amount of pressure occurring in the right side of your heart.

Pulmonary function tests

A person using a spirometer

SpirometerOpen pop-up dialog box

Spirometry and diffusion capacity. This test requires you to exhale quickly and forcefully through a tube connected to a machine that measures how much air your lungs can hold, and how quickly you can move air out of your lungs. It also measures how easily oxygen can move from the lungs into the bloodstream.

Oximetry. This simple test uses a small device placed on one of your fingers to measure the oxygen saturation in your blood. It may be done at rest or with activity to monitor the course and severity of lung disease.

Lung tissue analysis

Often, pulmonary fibrosis can be definitively diagnosed only by examining a small amount of lung tissue (biopsy) in a laboratory.


The tissue sample may be obtained in one of these ways:


Bronchoscopy. In this procedure, your doctor removes very small tissue samples — generally no larger than the head of a pin — using a small, flexible tube (bronchoscope) that's passed through your mouth or nose into your lungs. The risks of bronchoscopy are generally minor — most often a temporary sore throat and hoarseness from the bronchoscope — but the tissue samples are sometimes too small for an accurate diagnosis.

Bronchoalveolar lavage. In this procedure, your doctor injects about a tablespoon of salt water through a bronchoscope into a section of your lung, and then immediately suctions it out. The solution that's withdrawn contains cells from your air sacs. Although bronchoalveolar lavage samples a larger area of the lung than other procedures do, it may not provide enough information to diagnose pulmonary fibrosis.

Surgical biopsy. Although this is a more invasive procedure with potential complications, it's often the only way to obtain a large enough tissue sample to make an accurate diagnosis. While you are under general anesthesia, surgical instruments and a small camera are inserted through two or three small incisions between your ribs. The camera allows your surgeon to view your lungs on a video monitor while removing tissue samples from your lungs.


Treatment

The lung scarring that occurs in interstitial lung disease can't be reversed, and treatment will not always be effective in stopping the ultimate progression of the disease. Some treatments may improve symptoms temporarily or slow the disease's progress. Others help improve quality of life.


Because many of the different types of scarring disorders have no approved or proven therapies, clinical studies may be an option to receive an experimental treatment.


Medications

Intense research to identify treatment options for specific types of interstitial lung disease is ongoing. Based on currently available, scientific evidence, however, your doctor may recommend:


Corticosteroid medications. Many people diagnosed with interstitial lung diseases are initially treated with a corticosteroid (prednisone), sometimes in combination with other drugs that suppress the immune system. Depending on the cause of the interstitial lung disease, this combination may slow or even stabilize disease progression.

Medications that slow the progression of idiopathic pulmonary fibrosis. The medications pirfenidone (Esbriet) and nintedanib (Ofev) may slow the rate of disease progression. Treatment-related side effects may be significant. Talk through the pros and cons of these medications with your doctor.

Medications that reduce stomach acid. Gastroesophageal reflux disease (GERD) affects the majority of people with idiopathic pulmonary fibrosis and is associated with worsening lung damage. If you have symptoms of acid reflux, your doctor may prescribe GERD therapies that reduce stomach acid, including H-2-receptor antagonists or proton pump inhibitors such as lansoprazole (Prevacid 24HR), omeprazole (Prilosec OTC) and pantoprazole (protonix).